Brain and spinal cord, Churg-Strauss syndrome [LMP21018]

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Diagnostic Modality: 
Whole Slide Image
Disease Category: 
Diseases of the Immune System
Diagnosis: 
Churg-Strauss syndrome involving the CNS
Species: 
Human
Organ System: 
Central Nervous System
Gender: 
Male
Age: 
61 years
Diagnosis Code: 
ICD-10: M30.1
Slide Contributor Information
Gao, A., Munoz, D.

Keywords

Eosinophilic granulomatosis with polyangiitis, allergic granulomatous angiitis, vasculitis, infarct, stroke

Clinical History

61-year old man with bilateral lower extremity weakness and a T6 sensory level. Neuroimaging showed multiple ring enhancing lesions in the brain and a single lesion in the thoracic spinal cord. The patient also had peripheral eosinophilia.

Clinical Discussion

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) is a vasculitis of small and medium sized arteries. Lung, skin, and peripheral nerves are most commonly affected but it may involve the cardiovascular, gastrointestinal, renal, and central nervous systems. CNS involvement occurs in approximately 7% of cases and typically presents as infarct or hemorrhage. Microscopically, blood vessels show an eosinophil-rich inflammatory infiltrate in the vessel wall, often with fibrinoid necrosis. Granulomas are occasionally seen. Treatment is generally with steroids and other immunosuppressive agents.